Endomyocardial fibrosis in Sudan: clinical and echocardiographic features

Abstract

SummaryObjective:Endomyocardial fibrosis (EMF) is a rare disease and is often an underdiagnosed and forgotten cardiomyopathy. The objective of this study was to document the current frequency of EMF in Sudan by defining and selecting cases from patients attending the echocardiography laboratory. Additionally we aimed to create an EMF registry for Sudan.Methods:The study started in January 2007 and is on-going. All the patients attending our echocardiography clinics in four different hospitals in Khartoum, Sudan, were included. Transthoracic echocardiography was used as the main diagnostic and selection tool. The diagnosis of EMF was based on predefined criteria and definitions, and was further supported by additional clinical, ECG, laboratory and chest X-ray findings.Results:Out of 4 332 cases studied, 23 (0.5%) were found to have features of EMF. Females constituted 52% and the age range was 24 to 67 years. All patients presented with dyspnoea grades III–IV. Advanced heart failure with gross fluid overload was seen in 54% of cases and ascites was seen in 30%. EMF was biventricular in 53%, left ventricular in 29% and right ventricular in 18% of cases. Apical and ventricular wall fibrosis was found in all cases, followed by atrial enlargement, atrioventricular valve incompetence, ventricular cavity obliteration, restrictive flow pattern and pericardial effusion. Additional echocardiographic features are defined and discussed.Conclusion:Although a rare disease, cases of EMF can be identified in Sudan if a high index of suspicion is observed. New echocardiographic features of ventricular wall layering, endocardial fibrous shelf and endomyocardiopericarial fibrosis were identified and are discussed.

<p>Endomyocardial Fibrosis: Diagnosis and Management</p>

“…A further noticeable finding is the spread of the thick endocardium of the posterior left ventricular wall to engulf the posterior mitral valve leaflet with the formation of an endocardial fibrous shelf (EFS). 34 This autopsy finding was noted by Davies and is shown echocardiographically in Figure 2. Pericardial effusion is a common finding and is seen in 87% of patients who present with heart failure.…”

“…37 There are five diagnostic echocardiographic features of EMF; apical fibrosis, ventricular wall fibrosis, huge atrium, atrioventricular valve regurgitation and obliteration of ventricular cavity ( Figure 1A and B). 34 These features are commonly seen together in the same patient and are considered the prime diagnostic findings.…”

“…This feature merits the term endomyocardiopericarial fibrosis (EMPF) and is considered a new additional echocardiographic finding. 34 Three echocardiographic types of EMF are recognized; left ventricular which constituted 53% of cases (Figure 1), right ventricular seen in 18% of cases ( Figure 5A), and biventricular which was seen in 29% of cases ( Figure 5B).…”

“…Tense ascites is commonly seen in EMF and accounted for 30% in a series reported from Sudan. 34 It is associated with mild or no edema in some cases. Freers et al reported exudative content, unlike passive ascites seen in congestive failure, and the appearance of peritoneal fibrosis in some cases.…”

“…Chest X-ray findings were also nonspecific and showed cardiomegaly in 92% and pericardial effusion in 87% of patients. 34…”